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Developmental disorders of the vagina and vulva include many different structural problems that occur while the baby is developing in the mother's womb.
Abnormalities of the female vagina and vulva include:
Imperforate hymen -- The hymen is a thin tissue that partly covers the opening to the vagina. An imperforate hymen completely blocks the vaginal opening, so menstrual blood or mucus cannot flow out of the body. This often leads to painful swelling of the vagina. Sometimes the hymen has only a very small opening. This problem may not be discovered until puberty. Some baby girls are born without a hymen.
Vaginal abnormalities -- A baby girl may be born without a vagina or have the vaginal opening blocked by a layer of cells that are higher up in the vagina than where the hymen is. A missing vagina is most often due to Mayer-Rokitansky-Kuster-Hauser syndrome. In this syndrome, the baby is missing part or all of the internal reproductive organs (uterus, cervix, and fallopian tubes). Other abnormalities include having two vaginas or a vagina that opens into the urinary tract.
Problems with outer (external) genitals -- Developmental problems may cause the folds of tissue around the opening of the vagina to join together. This is called a fused labia. Other developmental problems may lead to a swollen clitoris or ambiguous genitalia.
Sanfilippo JS. Vulvovaginal and müllerian anomalies. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 554.
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